Aetiology and management of extrahepatic portal vein obstruction in children: King's College Hospital experience.
OBJECTIVE
To study a single-centre experience of the management of extrahepatic portal vein obstruction (EHPVO) in children during the last 3 decades.


METHODS
The medical records of 108 children (67 male, median age 4.75 years, range = 1 day-16.3 years) presenting with EHPVO between 1979 and 2005 were reviewed retrospectively.


RESULTS
Extended prothrombotic screening performed in 30 patients revealed low protein C activity (6 patients), low free protein S (2), and a positive lupus anticoagulant (1); factor V Leiden mutations and the JAK2V617F mutation were not identified. Associated congenital anomalies were found in 26 of the 108 children (24%). Clinical presentation included splenomegaly in 98 (91%) and ascites in 3 (3%). Elevation of liver enzymes and prolonged international normalized ratio were seen in 13 (12%) and 14 (13%) children, respectively. Haematological parameters of hypersplenism were present in 13 (12%). Bleeding occurred in 83 (77%) patients with a median age of 4.58 (0.02-16.37) years. On first endoscopy, oesophageal varices were present in 92 patients; of those subjects, 70 (76%) received sclerotherapy, 5 (5%) had band ligation, and 16 (17%) received both. Complications of endoscopy occurred in 34 (37%) patients: oesophageal ulcers in 16, oesophageal stricture in 10, both in 7, and erosive gastritis in 1. Seventeen (16%) children underwent shunt surgery for uncontrolled bleeding at a median age of 9.7 (5.2-23.7) years.


CONCLUSIONS
The aetiology of EHPVO in the majority of patients remains unknown. Sclerotherapy and banding are effective treatments for bleeding varices with good long-term outcome. Procoagulant state is an infrequent cause of EHPVO in children.
Abd El-Hamid
Nehal
N
Paediatric Liver Centre, King's College London School of Medicine at King's College Hospital, London, UK.
Taylor
Rachel M
RM
Marinello
Daniela
D
Mufti
G J
GJ
Patel
Raj
R
Mieli-Vergani
Giorgina
G
Davenport
Mark
M
Dhawan
Anil
A
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004932:Esophageal and Gastric Varices / Q000097:blood / Q000209:etiology / Q000601:surgery*; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000601:surgery / Q000628:therapy; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D019934:International Normalized Ratio; D008297:Male; D008499:Medical Records; D010976:Platelet Count; D011169:Portal Vein / Q000473:pathology*; D011486:Protein C / Q000378:metabolism; D012189:Retrospective Studies; D015911:Sclerotherapy / Q000379:methods; D005723:gamma-Glutamyltransferase / Q000097:blood
D011486:Protein C; D005723:gamma-Glutamyltransferase